Mast cell disease and the spleen
- The spleen removes old or damaged red blood cells.
- It holds a supply of blood cells that can be released in case of hemorrhage.
- It recycles iron from old red blood cells.
- It filters out bacteria.
- When the bone marrow is not producing enough blood cells, the spleen can make red and white blood cells.
- Swelling of the spleen is called splenomegaly.
- If you can feel the spleen, it is at least twice its normal size.
- Swelling of the spleen may have no symptoms, but it can cause referred pain, especially in the shoulder.
- If the bone marrow is not producing enough blood cells, the spleen can become swollen because of the stress of making so many cells.
- Swelling of the spleen is pretty common in blood disorders.
- Swelling of the spleen is a B finding in SM. If you have two B findings, you have smoldering systemic mastocytosis.
- Hypersplenism is when the spleen is working too hard.
- Hypersplenism with splenomegaly is a C finding, which means you have aggressive systemic mastocytosis.
- Hypersplenism is indicated by swelling of the spleen, reduction in red blood cells, platelets or granulocytes, increased cell production by the bone marrow, and probable resolution if the spleen is removed.
- Hypersplenism can cause additional complications like ascites (free fluid in the abdomen) and port hypertension (increased pressure in the vein between the GI tract and spleen).
- Portal hypertension and ascites are also C findings.
- Removal of the spleen can improve prognosis.
- Patients with no spleen are more susceptible to certain types of infections.
- In 2010, the US spent an average of $8233 per patient.
- The US spends 17.6% of GDP on healthcare, compared to other developed nations which average 9.5%.
- The US has fewer practicing physicians and fewer hospital beds.
- In the US, we spend 2.5x more on ambulatory care, such as walk-in clinics and emergency rooms.
- We do not have enough PCPs in the US.
- Administrative cost per patient is much higher in the US, partly due to not having electronic records.
- The US performs more tests than other countries.
- Five year survival rates for many cancers are best in the US.
- Access to newer medications and treatments is more readily available in the US.
- The human body keeps fluids in two spaces called compartments.
- The first compartment is inside cells.
- The second compartment is outside of the cells, like in the tissues around the cells.
- The second compartment holds 40% of fluids.
- Third spacing is when your fluids collect outside of one of these two compartments.
- Fluid in a third space is unusable by the body.
- Ascites, pulmonary edema and angioedema are all forms of third spaces.
- Third spacing can compress structures around the fluid.
- Third spacing can sometimes affect organ function.
- Third spacing can cause the fluid level in the circulatory system to drop.
- People with frequent third spacing often have symptoms of dehydration.
- Third spacing can occur due to anaphylaxis or mast cell activation.
- IV fluids are helpful for some patients who have third spacing.
Sex and chronic illness series: Vaginal pain
- Vulvodynia is vulvar pain without an obvious cause.
- Vulvodynia can cause burning, stinging or sharp pain in the vulva, labia and vaginal opening.
- Vaginal penetration can be painful with vulvodynia.
- Any pressure on the genital area, including sitting, can be painful with vulvodynia.
- Vulvar vestibulitis is a subset of vulvodynia found in 10-15% of women who receive regular gynecological care.
- Women with a chronic pain disorder like interstitial cystitis or fibromyalgia are three times more likely to have vulvodynia.
- If they have more than one pain disorder, their risk increases over five fold.
- 73% of vulvodynia have no other known chronic pain disorder.
- Mast cells have been linked to sexual pain disorders.
- Allergic reactions or mast cell degranulation may cause vulvodynia.
- Vulvodynia patients have high numbers of degranulated mast cells in biopsies.
- Wearing cotton underwear and avoiding scented products can help vulvodynia.
- Oxalate rich diets can irritate vulvodynia but do not cause it.
- Creams with cromolyn are effective for vulvodynia pain.
- Physical therapy and biofeedback can help pelvic floor dysfunction.
- If penetration is painful, dilation can be helpful.
- A vestibulectomy is a last resort and is about 80% effective.
- About 116 million people in the US live with chronic pain.
- Bacteria are categorized by the way they are shaped and grouped, and how they derive energy.
- Bacteria are also categorized by their reaction to a test called a Gram stain.
- Antibiotics inhibit the growth of or kill microbes, including bacteria.
- Medications that kill fungi are called antimycotics or antifungals.
- Antivirals kill viruses, but viruses are not technically alive.
- Some antibiotics are only effective against specific types of bacteria. These are called “narrow spectrum” antibiotics.
- Some antibiotics are effective against many types of bacteria. These are called “wide spectrum” antibiotics.
Antibiotic resistance (part two)
- It took less than five years for antibiotic resistance to develop to penicillin, the first widely used antibiotic.
- Using antibiotics unnecessarily contributes to antibiotic resistance.
- Antibiotics do not treat viral infections.
- If you get repeat, frequent infections of the same nature, you need to see an infectious disease specialist.
- Less than 1% of mastocytosis cases are MCL.
- It can occur in a patient who previously had mastocytosis or with no history of mast cell disease.
- MCL patients have bone marrow infiltration of at least 20% mast cells and infiltration of internal organs.
- CKIT D816V mutation is not always present.
- Median survival is six months.
- Swelling of spleen and liver are common in MCL patients.
- Only 1/3 of MCL patients have skin involvement.
- In 25% of MCL cases, CD25 is not expressed; in 33%, neither CD25 nor CD2 is expressed.
- Average age of diagnosis with MCL is 52 years old.
- 27% of patients had a history of mastocytosis.
- Only four cases recorded of children with mastocytosis evolving into MCL.
- In one case, a child with a mastocytoma developed MCL.
- Treatment is largely experimental.
- Seven patients have received stem cell transplants. All have died in less than three years.
Diagnosis of mast cell diseases
- Cutaneous mastocytosis, including UP, TMEP and DCM (diffuse cutaneous mastocytosis), is diagnosed by biopsy.
- Mastocytoma can also be diagnosed by biopsy, but urtication on touching is usually diagnostic.
- SM has one major and four minor criteria. You must meet one major and one minor, or three minor criteria for diagnosis.
- The most common method for diagnosing SM is bone marrow biopsy, but biopsy from any non-skin organ may meet criteria.
- SM patients are biopsy negative 1/6 of the time.
- The blood test for the CKIT D816V mutation is not always reliable. Bone marrow is more reliable.
- Serum tryptase criterion for SM is baseline tryptase, not reaction tryptase.
- There are three criteria for SM called B findings that show disease progression. If you have two or more B findings, you have smoldering systemic mastocytosis.
- There are five criteria for SM called C findings that show aggressive disease. If you have one or more C findings, you have aggressive systemic mastocytosis.
- Mast cell leukemia is usually diagnosed through bone marrow biopsy.
- Mast cell sarcoma is a very aggressive tumor that converts to mast cell leukemia. It is diagnosed by biopsy.
- Mast cell sarcoma is different from mastocytoma.
- Mast cell activation syndrome (MCAS) is diagnosed by evidence of mediator release, presence of mediator release symptoms, response to mast cell medications and absence of another cause for mast cell activation.